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以睑板腺炎、巩膜炎、葡萄膜炎和乳头状膀胱肿瘤为表现的非典型肉芽肿性多血管炎:病例报告及文献复习

Atypical Granulomatosis with Polyangiitis Presenting with Meibomitis, Scleritis, Uveitis and Papillary Bladder Tumor: A Case Report and Literature Review.

作者信息

Kojima Takashi, Dogru Murat, Shimizu Eisuke, Yazu Hiroyuki, Takahashi Aya, Shimazaki Jun

机构信息

Department of Ophthalmology, Keio University School of Medicine, Tokyo 160-8582, Japan.

Department of Ophthalmology, Tokyo Dental College Ichikawa General Hospital, Chiba 272-8513, Japan.

出版信息

Diagnostics (Basel). 2021 Apr 9;11(4):680. doi: 10.3390/diagnostics11040680.

DOI:10.3390/diagnostics11040680
PMID:33918928
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8069415/
Abstract

Granulomatosis with polyangiitis (GPA) presents with a variety of systemic findings, sometimes with ocular findings initially, but is often difficult to diagnose at an early stage. An 85-year-old male had complaints of ocular dryness and redness and was diagnosed with meibomian gland dysfunction with meibomitis. Despite an initial treatment with topical steroid and antibiotics, the meibomitis did not improve and the left eye developed scleritis and iridocyclitis. The patient was administered topical mydriatics and oral steroids. During follow-up, the patient developed left hearing difficulty and reported a darker urine. Urinalysis revealed microscopic hematuria. A blood test showed an elevated erythrocyte sedimentation rate, positivity for perinuclear anti-neutorophil cytoplasmic antibody, and elevations in blood urea nitrogen and serum creatinine. Nasal mucosal biopsy showed a non-necrotizing granulomatous inflammation. Renal biopsy revealed focal glomerulosclerosis. Cystoscopy and bladder wash followed by a planned transurethral resection revealed atypical cells and apical papillary tumors which were resected. Iridocyclitis and scleritis responded well to oral prednisolone with 0.1% topical betamethasone and prednisolone ointment. The patient is tumor free with no recurrences 24 months after resection. GPA may present atypically with meibomian gland dysfunction without showing representative clinical findings. Early detection and treatment are essential for visual recovery.

摘要

肉芽肿性多血管炎(GPA)可表现出多种全身症状,有时最初会出现眼部症状,但早期往往难以诊断。一名85岁男性主诉眼干、眼红,被诊断为睑板腺功能障碍合并睑板炎。尽管最初使用了局部类固醇和抗生素治疗,但睑板炎并未改善,左眼出现了巩膜炎和虹膜睫状体炎。给予患者局部散瞳剂和口服类固醇治疗。在随访过程中,患者出现左耳听力下降,并报告尿液颜色加深。尿液分析显示镜下血尿。血液检查显示红细胞沉降率升高、核周抗中性粒细胞胞浆抗体阳性,血尿素氮和血清肌酐升高。鼻黏膜活检显示非坏死性肉芽肿性炎症。肾活检显示局灶性肾小球硬化。膀胱镜检查和膀胱冲洗后计划行经尿道切除术,发现非典型细胞和顶端乳头状肿瘤并进行了切除。虹膜睫状体炎和巩膜炎对口服泼尼松龙联合0.1%局部倍他米松和泼尼松龙眼膏反应良好。患者在切除术后24个月无肿瘤复发。GPA可能以非典型的睑板腺功能障碍形式出现,而不表现出典型的临床症状。早期发现和治疗对于视力恢复至关重要。

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